The objective of the proposed work is to investigate the mechanism of neuronal and glial degeneration and demyelination, which are the major neuropathological changes observed in varieties of metabolic neurological disorders in infancy and childhood. Neurological mutant mice with hypomyelination (quaking and with defect in copper metabolism (brindled, mottled) and other experimentally induced animal models are used in the investigations, which are mainly morphological (E.M., L.M. and Golgi preparation).